Perforating Disorders


(Figure 3.38)
  • Group of disorders characterized by transepidermal elimination of altered dermal connective tissue or keratin
  • See Table 3 -19
Figure 3.38 Acquired perforating dermatosis (Courtesy of Dr. Paul Getz)
Figure 3.38
Acquired perforating dermatosis
(Courtesy of Dr. Paul Getz)
   
 
Table 3-19 Perforating Disorders
 Disease Description Histology Associations
Acquired perforating dermatosis
(Kyrle’s disease)
(Acquired reactive perforating collagenosis)
Intensely pruritic umbilicated papules with keratinous plugs involving extensor limbs, less common on trunk/face, + Köebner phenomenon
Cup-shaped invagination of epidermis with hyperkeratotic plug containing inflammatory debris and collagen fibers, vertically oriented collagen extruded into plug (in dermis)
Diabetes or renal failure (many patients on hemodialysis), some cases related to malignancy
Inherited reactive
perforating collagenosis
Similar lesions but occur in childhood
Similar histology to acquired form
None
Elastosis perforans
serpiginosa
(EPS)
Skin-colored to red papules in serpiginous, linear or arciform pattern over upper extremities or neck, may measure several centimers in diameter
↑↑ Amount of elastic fibers in papillary dermis appearing clumped and ‘clutched’ by epidermis (hyperplastic); elastic fibers and inflammatory cells extruded via granulomatous reaction
Associated with:


{MADD PORES:Marfan’s, Acrogeria, Down syndrome,
D-
Penicillamine, Pseudoxanthoma elasticum, Osteogenesis imperfecta, Rothmund-Thomson, Ehlers Danlos, Scleroderma}
Perforating folliculitis
Keratotic follicular papules commonly over extensor surfaces
Involved hair follicle shows disruption of lateral wall; parakeratotic plug consists of collagen, elastic fibers and inflammatory cells
Chronic renal failure and diabetes
Perforating
periumbilical calcific
elastosis
Keratotic papules involving the abdomen
Transepidermal elimination of calcified elastic fibers
African-American women,
multiparity